Infant Sickle Cell Program

Sickle cell disease affects many families; in fact, one in 500 African-American children born in the United States will be diagnosed with sickle cell disease, making it the most commonly diagnosed disease in newborn screenings. Sickle cell disease is caused by a change in the genes for hemoglobin, which carries oxygen inside the red blood cells. This change causes the red blood cells to take on a sickle or crescent shape, which tend to clog small blood vessels, blocking blood flow and decreasing the amount of oxygen delivered to the tissues and organs, leading to the complications associated with sickle cell disease, like pain crisis, acute chest syndrome, and stroke.

When a family visits the Infant Sickle Cell Program, expect 10-15 minutes of education about sickle cell disease and common complications of the disease in young children. This is followed by 10-15 minutes of education about topics that are important to families of sickle cell patients. Topics include:

• Hydroxyurea use
• Bone marrow transplantation
• What to expect in the emergency department
• How to interact with children to improve their development
• Genetics of sickle cell disease
• Research

Children’s National’s experts are constantly working toward better outcomes for sickle cell disease patients and families. Researchers participated in the Baby HUG study which examined risks and benefits of treating young children (9-18 months of age) with hydroxyurea, a medicine used to ease the complications of sickle cell disease. Follow-up Baby HUG studies are continuing to study these children to determine how hydroxyurea affects them. They are also working to understand more about the individual response to pain in order to create individualized pain plans for sickle cell patients and are studying bone marrow transplantation for children who don’t have a sibling match for transplant as well as ways to identify sickle cell infants who are the highest risks for complications later in life.